Alexiou C, Mahmoud H, Al-Khaddour A, Gnanapragasam J, Salmon A P, Keeton B R, Monro J L
Department of Cardiac Surgery, The General Hospital, Southampton, United Kingdom.
Ann Thorac Surg. 2001 Feb;71(2):494-500. doi: 10.1016/s0003-4975(00)02444-9.
The purpose of this study was to evaluate the early and late outcome after repair of tetralogy of Fallot in the first year of life.
Between 1974 and 2000, 89 consecutive infants with a mean age of 6.3 +/- 2.6 months (range, 15 days to 12 months) underwent repair of tetralogy of Fallot (ventricular septal defect and pulmonary stenosis) by one surgeon (J.L.M.). Three infants had previous palliative operations. Sixty-seven procedures were urgent or emergency. A transannular patch was inserted in 69 patients (77.5%). Follow-up was complete, averaging 13.4 +/- 5.6 years (range, 0 to 25.4 years).
There was one operative death (1.1%). Mean right ventricular to left ventricular pressure ratio postoperatively was 0.4 +/- 1.1 (in 79 patients, < 0.5). Fourteen patients underwent reoperations or reinterventions. There were no reoperations for residual or recurrent ventricular septal defect. Kaplan-Meier freedom from reoperation or reintervention for any cause at 20 years was 85% +/- 4.4%, for relief of right ventricular outflow tract obstruction it was 94% +/- 3.1%, and for pulmonary valve replacement this was 95.4% +/- 2.6%. Use of a transannular patch did not significantly affect the need for reoperation or reintervention. There was one late death (leukemia). Kaplan-Meier 20-year survival was 97.8% +/- 1.9%. On latest echocardiography, 42 patients had moderate pulmonary regurgitation, 4 had a right ventricular outflow tract gradient more than 40 mm Hg, and 86 had good biventricular function. Twelve-lead electrocardiography was performed in all and 24-hour electrocardiography in 61 patients. One patient (1.1%) exhibited late recurrent ventricular tachycardia requiring implantation of a defibrillator. The remaining 86 patients are in New York Heart Association class I with none of them receiving antiarrhythmic medications.
These data strongly support the concept of early repair of tetralogy of Fallot. It is associated with an acceptable operative risk and a low incidence of significant arrhythmias, and provides long-term survival similar to that observed in the general population. Late complications may, however, develop, and long-term follow-up for their early recognition is essential.
本研究的目的是评估出生后第一年法洛四联症修复术后的早期和晚期结果。
1974年至2000年期间，89例平均年龄为6.3±2.6个月（范围15天至12个月）的连续婴儿由一名外科医生（J.L.M.）进行了法洛四联症（室间隔缺损和肺动脉狭窄）修复术。3例婴儿曾接受过姑息手术。67例手术为急诊或紧急手术。69例患者（77.5%）植入了跨环补片。随访完整，平均随访时间为13.4±5.6年（范围0至25.4年）。
有1例手术死亡（1.1%）。术后右心室与左心室压力比值平均为0.4±1.1（79例患者中，<0.5）。14例患者接受了再次手术或再次干预。没有因残余或复发性室间隔缺损而进行再次手术的情况。20年时因任何原因无需再次手术或再次干预的Kaplan-Meier生存率为85%±4.4%，因缓解右心室流出道梗阻的生存率为94%±3.1%，因肺动脉瓣置换的生存率为95.4%±2.6%。使用跨环补片对再次手术或再次干预的需求没有显著影响。有1例晚期死亡（白血病）。20年Kaplan-Meier生存率为97.8%±1.9%。在最近一次超声心动图检查中，42例患者有中度肺动脉反流，4例患者右心室流出道压差超过40 mmHg，86例患者双心室功能良好。所有患者均进行了12导联心电图检查，61例患者进行了24小时心电图检查。1例患者（1.1%）出现晚期复发性室性心动过速，需要植入除颤器。其余86例患者纽约心脏协会心功能分级为I级，均未接受抗心律失常药物治疗。
这些数据有力地支持了法洛四联症早期修复的概念。它与可接受的手术风险和显著心律失常的低发生率相关，并提供了与普通人群相似的长期生存率。然而，可能会出现晚期并发症，对其进行早期识别的长期随访至关重要。
